{"orpha":228299,"name":"Mid-dermal elastolysis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, acquired, dermis elastic tissue disease characterized by asymptomatic, well-demarcated, symmetric patches and/or plaques of finely wrinkled skin arranged parallel to skin cleavage lines (type I), associated with perifollicular papular protrusions (type II) or with persistent reticular erythema (type III), occurring predominantly on the shoulders, trunk, back, and proximal extremities, associating, on histopathology, a selective loss of elastic tissue in the midreticular dermis. Erythema and/or urticaria may or may not precede wrinkly lesions.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-228299/","orphanet_url":"https://www.orpha.net/en/disease/detail/228299","names":{"fr":{"name":"Elastolyse du derme moyen","definition":"Anomalie acquise de l'élasticité du derme rare, caractérisée par des taches et/ou des plaques asymptomatiques, bien délimitées et symétriques de peau fripée, disposées parallèlement aux lignes de clivage de la peau (type I), associées à des protubérances papulaires périfolliculaires (type II) ou à un érythème réticulaire persistant (type III), touchant principalement les épaules, le tronc, le dos et les parties proximales des extrémités, associées à une perte sélective de l'élasticité du derme réticulaire moyen, visible à l'examen histologique. Un érythème et/ou une urticaire peuvent précéder ou non les lésions sous forme de plaques de peau fripée."},"de":{"name":"Mid-dermale Elastolyse","definition":"Eine seltene, erworbene Erkrankung des elastischen Gewebes der Dermis. Sie ist gekennzeichnet durch asymptomatische, gut abgegrenzte, symmetrische Flecken und/oder Plaques fein faltiger Haut, die entweder parallel zu den Spaltlinien der Haut angeordnet sind (Typ I), verbunden sind mit perifollikulären papulösen Ausstülpungen (Typ II) oder mit einem persistierenden retikulären Erythem (Typ III) einhergehen, das vorwiegend an Schultern, Rumpf, Rücken und proximalen Extremitäten auftritt und histopathologisch mit einem selektiven Verlust an elastischem Gewebe in der mittleren retikulären Dermis einhergeht. Den faltigen Läsionen können Erytheme und/oder Urtikaria vorausgehen."},"es":{"name":"Elastolisis de la dermis media","definition":"Es una enfermedad adquirida y poco frecuente del tejido elástico de la dermis caracterizada por manchas y/o placas simétricas asintomáticas y bien delimitadas de piel ligeramente arrugada dispuestas de forma paralela a las líneas de menor tensión de la piel (tipo I), asociadas a protuberancias papulares perifoliculares (tipo II) o a eritema reticular persistente (tipo III), que afecta predominantemente a los hombros, el tronco, la espalda y las extremidades proximales, asociando en la histopatología una pérdida selectiva de tejido elástico en la dermis medio-reticular . El eritema y/o la urticaria pueden preceder a las lesiones de piel arrugada."},"it":{"name":"Elastolisi del derma medio","definition":"È una malattia acquisita rara del tessuto elastico del derma, caratterizzata da macchie e/o placche asintomatiche, simmetriche, ben delimitate, composte da cute che mostra plicature sottili organizzate parallelamente rispetto alle linee di clivaggio della cute (tipo I), associate a protrusioni papulari perifollicolari (tipo II) o ad eritema reticolare persistente (tipo III), localizzate prevalentemente sulle spalle, sul tronco, sulla schiena e sulla porzione prossimale degli arti. Gli esami istopatologici evidenziano la perdita selettiva del tessuto elastico nel derma medio-reticolare. Le plicature della cute possono essere precedute da eritema e/o orticaria."},"nl":{"name":"Mid-dermale elastolyse","definition":"Een zeldzame, verworven ziekte van het elastisch weefsel van de dermis, gekarakteriseerd door asymptomatische, goed begrensde, symmetrische plekken en/of plaques van fijn gerimpelde huid, die parallel gerangschikt zijn aan de splijtlijnen van de huid (type I), geassocieerd zijn met perifolliculaire papulaire uitsteeksels (type II) of geassocieerd zijn met persisterend reticulair erytheem (type III), die voornamelijk voorkomen op de schouders, de romp, de rug, en de proximale ledematen, en die histopathologisch associëren met een selectief verlies van elastisch weefsel in de midreticulaire dermis. Erytheem en/of urticaria kunnen al dan niet voorafgaan aan de rimpelige letsels."},"pt":{"name":"Elastólise da derme média"},"pl":{"name":"Elastoliza śródskórna"},"cs":{"name":"Mid-dermální elastolýza"},"uk":{"name":"Серньодермальний еластоз","definition":"A rare, acquired, dermis elastic tissue disease characterized by asymptomatic, well-demarcated, symmetric patches and/or plaques of finely wrinkled skin arranged parallel to skin cleavage lines (type I), associated with perifollicular papular protrusions (type II) or with persistent reticular erythema (type III), occurring predominantly on the shoulders, trunk, back, and proximal extremities, associating, on histopathology, a selective loss of elastic tissue in the midreticular dermis. Erythema and/or urticaria may or may not precede wrinkly lesions."},"zh":{"name":"皮肤中度弹性组织离解","definition":"一种罕见的获得性真皮弹性组织疾病，其特征是无症状、界限清楚、对称的斑块和/或平行于皮纹的细小褶皱斑块（I型），伴有周围滤泡丘疹突起（II型）或持续性网状红斑（III型），主要发生在肩部、躯干、背部和四肢近端，在组织病理学上与网状真皮中的弹性组织选择性丧失有关。红斑和/或荨麻疹的出现可能会或可能不会先于皱纹病变。"},"tr":{"name":"Orta dermal elastoliz"}},"xrefs":[{"source":"ICD-10","code":"L98.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EE41.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1379952112"},{"source":"MONDO","code":"0016449","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4728147","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10081688","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":228221,"name":"Acquired dermis elastic tissue disorder with decreased elastic tissue","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}